Article
The lncRNA H19 alleviates muscular dystrophy by stabilizing dystrophin.
Nature cell biology - 1 Nov 2020
Zhang Yaohua, Li Yajuan, Hu Qingsong, Xi Yutao, Xing Zhen, Zhang Zhao, Huang Lisa, Wu Jianbo, Liang Ke, Nguyen Tina K, Egranov Sergey D, Sun Chengcao, Zhao Zilong, Hawke David H, Li Jin, Sun Deqiang, Kim Jean J, Zhang Ping, Cheng Jie, Farida Abid, Hung Mien-Chie, Han Leng, Darabi Radbod, Lin Chunru, Yang Liuqing
Abstract excerpt
Dystrophin proteomic regulation in muscular dystrophies (MDs) remains unclear. We report that a long noncoding RNA (lncRNA), H19, associates with dystrophin and inhibits E3-ligase-dependent polyubiquitination at Lys 3584 (referred to as Ub-DMD) and its subsequent protein degradation. In-frame deletions in BMD and a DMD non-silent mutation (C3340Y) resulted in defects in the ability of the protein to interact with...
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